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Presentation of cutaneous mastocytosis in 173 children
1New Children's Hospital, Sydney, New South Wales, Australia. dreg1@canc.rpa.cs.nsw.gov.au
Insights
This study reviewed 173 pediatric cutaneous mastocytosis cases, finding most cases appeared by six months. Symptoms like flushing and blistering varied by mastocytosis type, with diffuse cutaneous mastocytosis showing the highest incidence.
Area of Science:
- Pediatric Dermatology
- Cutaneous Disorders
- Mast Cell Biology
Background:
- Cutaneous mastocytosis (CM) is a rare condition involving mast cell accumulation in the skin.
- It presents in various forms, including mastocytomas, urticaria pigmentosa, and diffuse CM.
- Early diagnosis and understanding of CM subtypes are crucial for management.
Purpose of the Study:
- To retrospectively analyze clinical features and presentation of cutaneous mastocytosis in a pediatric cohort.
- To describe the incidence of specific symptoms like flushing and blistering across different CM subtypes.
- To document rare presentations, such as palmar mastocytoma.
Main Methods:
- Retrospective chart and clinical photograph review of 173 children diagnosed with CM.
- Data collection included patient demographics, CM subtype, age of onset, and presence of symptoms.
- Descriptive statistical analysis of the collected data.
Main Results:
- The study included 173 children (98 males, 75 females) with CM, with 87% presenting before six months of age.
- Mastocytomas (51%) and urticaria pigmentosa (47%) were the most common subtypes; three had diffuse CM.
- Flushing and blistering were observed in varying percentages across subtypes, with diffuse CM exhibiting 100% incidence for both symptoms. A rare palmar mastocytoma was noted.
Conclusions:
- Cutaneous mastocytosis in children predominantly appears early in infancy.
- Symptom presentation, including flushing and blistering, is highly variable and subtype-dependent.
- This review highlights the diverse clinical spectrum of pediatric CM and the importance of detailed case documentation.
Abstract:
This is a retrospective review of the case files and clinical photographs of 173 children diagnosed with cutaneous mastocytosis by a dermatologist in an exclusively paediatric practice. Of the 98 males and 75 females, 51% had mastocytomas, 47% had urticaria pigmentosa and three patients had diffuse cutaneous mastocytosis. Of these cases, 87% first appeared prior to or at 6 months of age. Flushing occurred in 26% of urticaria pigmentosa cases, 29% of mastocytomas and 100% of diffuse cutaneous mastocytosis. Blistering was noted in 23% of urticaria pigmentosa, 31% of mastocytomas and 100% of diffuse cases. Neither symptom was noted in 59% of urticaria pigmentosa and 49% of mastocytomas. There were three cases with a positive family history. The finding of a palmar mastocytoma has only once been previously reported. Illustrated descriptions of our cases are provided.